Health Guide

Pegvaliase-pqpz (By injection)

Pegvaliase-pqpz (peg-VAL-i-ase - pqpz)

Lowers phenylalanine levels in the blood in patients with phenylketonuria (PKU).

Brand Name(s):


There may be other brand names for this medicine.

When This Medicine Should Not Be Used:

This medicine is not right for everyone. Do not use it if you had an allergic reaction to pegvaliase-pqpz, unless your doctor tells you otherwise.

How to Use This Medicine:


  • Your doctor will prescribe your exact dose and tell you how often it should be given. This medicine is given as a shot under your skin.
  • Your doctor will give you the first dose of this medicine at the beginning and when restarting the medicine after you had an allergic reaction to the injection. You will also need to stay for at least 60 minutes to check for serious unwanted effects.
  • You may be taught how to give your medicine at home. Make sure you understand all instructions before giving yourself an injection. Do not use more medicine or use it more often than your doctor tells you to.
  • You will be shown the body areas where this shot can be given. Use a different body area each time you give yourself a shot. Keep track of where you give each shot to make sure you rotate body areas. Do not inject into skin areas that are tender, red, bruised, hard, burned, inflamed, or has scars, birthmarks, rashes, moles, or tattoos.
  • If more than one injection is needed, you may inject into the body area at least 2 inches away from each other. The second injection site can be on the same or a different part of the body.
  • Allow the medicine to warm to room temperature for 30 minutes before you use it. Do not warm it in any other way.
  • Check the liquid in the prefilled syringe. It should be clear or colorless to slightly yellow. Do not use it if it is cloudy, discolored, or has particles in it. Do not shake.
  • Use a new needle and syringe each time you inject your medicine.
  • You may also take other medicines (including an allergy medicine, fever medicine, or stomach medicine) before using the injection to prevent serious unwanted effects. You may also have your caregiver or family member stay with you for at least 60 minutes after injecting each dose to check for these effects.
  • This medicine should come with a Medication Guide. Ask your pharmacist for a copy if you do not have one.
  • Missed dose: If you miss a dose of this medicine, skip the missed dose and go back to your regular schedule. Do not double doses.
  • If you store this medicine at home, keep it in the refrigerator. Do not freeze. You may also keep this medicine at room temperature for up to 30 days. Do not return it to the refrigerator once you keep it at room temperature.

Drugs and Foods to Avoid:

Ask your doctor or pharmacist before using any other medicine, including over-the-counter medicines, vitamins, and herbal products.

Warnings While Using This Medicine:

  • Tell your doctor if you are pregnant or breastfeeding.
  • This medicine may cause serious allergic reactions. Always have an epinephrine autoinjector pen with you. Talk to your doctor about this.
  • Your doctor will check your progress and the effects of this medicine at regular visits. Keep all appointments.
  • Keep all medicine out of the reach of children. Never share your medicine with anyone.

Possible Side Effects While Using This Medicine:

Call your doctor right away if you notice any of these side effects:

  • Allergic reaction: Itching or hives, swelling in your face or hands, swelling or tingling in your mouth or throat, chest tightness, trouble breathing

If you notice these less serious side effects, talk with your doctor:

  • Headache, dizziness
  • Joint pain
  • Nausea, vomiting, stomach pain
  • Pain, itching, burning, swelling, or a lump under your skin where the shot was given
  • Tiredness

If you notice other side effects that you think are caused by this medicine, tell your doctor

Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088

Last Updated: 6/18/2019
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